AB1478 THERAPEUTIC TACTICS OF IDIOPATHIC LOBULAR PANNICULITIS
Authors: G. Koilubaeva, O. Egorova, A. Bolotbekova, A. Turatbekova, G. Syunbai K
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Idiopathic lobular panniculitis (ILP) or Weber-Christian disease is a rare understudied disease, which manifests itself in recurrent <i>subcutaneous fat</i> necrosis often involving damage of the internals. Treatment of the disease is not standardized, and as a matter of fact it is provided empirically. <h3>Objectives</h3> to assess the efficiency of therapy in case of various forms of ILP. <h3>Methods</h3> We studied 34 patients (30 women, 4 men) with ILP, age 30-74, duration of disease 7.4±2.3 years. Besides general clinical examination we determined the level of <i>α1-antitrypsin,</i> amylase, lipase, <i>ferritin, creatine phosphokinase,</i> made chest CT scan <i>and carried out the pathomorphological study</i> of <i>skin biopsy specimen taken from the area of a node.</i> The following forms of the disease were identified: nodular form (NF) (14 patients), plaque form (PF) (9 patients), mesenteric form (MF) (5 patients) and infiltrative form (IF) (6 patients). In all cases the lobular panniculitis was verified by means of histopathological examination. All patients took glucocorticoids (GC), average daily dose of 12.8 ± 1.6 mg, including 8.4± 1.7 mg (for NF), 17.6± 3.2 mg (for PF), 8,6 ± 1,6 mg (for МF), 23.6 ± 5.3 mg (for IF). In case of NF, we additionally prescribed Plaquenil, the dose of 600 mg per day (8 patients), and Mycophenolate Mofetil 1.5-2 g per day (6 patients); in case of PF – Cyclophosphamide (Cph), the dose of 200 mg per week (4 patients) or Mycophenolate Mofetil (1.5-2 g per day, 5 patients); in case of МF – Mycophenolate Mofetil (1.5-2 g per day, 5 patients). Patients with IF took Cph (200 mg per week). In case there was no effect after 3 months, we prescribed Mycophenolate Mofetil (1.5-2 g per day). With an unsatisfactory effect, after 3 months, genetically engineered alpha-TNF antagonist drugs were used. On average, the observation period was 12 months. <h3>Results</h3> The treatment made it possible to achieve a significant positive dynamics in patients with NF, PF and МF: the body temperature got back to normal and there was regression of indurations (26 patients), joint pain intensity was reduced (16 patients), laboratory data on inflammatory intensity revealed decrease (24 patients). Average daily dose of GC was reduced to 4.5±0.7 mg and 11.4±1.1 mg, respectively. In general, a significant improvement was evident in all patients with NF and МF, in 75 % of patients with PF. In case of IF an average daily dose was increased to 25.4±1.7 mg per day. In all patients of this group, an unsatisfactory effect was noted, which required discussion of the appointment of genetically engineered biological therapy. The tolerability of treatment was regarded as good, no adverse events were noted. After 14 weeks, 3 patients had a syndrome of "eluding" the effect and IL-1 inhibitor drugs were prescribed for the first time. <h3>Conclusion</h3> To work out dosages and treatment schedules for patients with ILP further studies are required. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.